Diagnosis and treatment of cystic fibrosis

C Koch, N Høiby

79 Citationer (Scopus)

Abstract

This review discusses some diagnostic aspects of cystic fibrosis (CF) including direct mutational analysis. Treatment of major disease manifestations is discussed in more detail with an emphasis on lung disease, in particular chronic infection with Pseudomonas aeruginosa which is responsible for the majority of excess morbidity and mortality. Centralised care and aggressive antimicrobial treatment have led to increased life expectancy and this may be even further increased by the demonstration that chronic P. aeruginosa infection may be prevented, or at least postponed for many years in a majority of patients. Adjunct treatment such as the use of local and systemic anti-inflammatory agents and inhalation of human recombinant DNase are also briefly touched upon. It is emphasised that important questions concerning the link(s) between the mutated gene and lung disease are still missing but that current research raises hope of a more causal treatment in the near future.

OriginalsprogEngelsk
TidsskriftRespiration; international review of thoracic diseases
Vol/bind67
Udgave nummer3
Sider (fra-til)239-47
Antal sider9
ISSN0025-7931
DOI
StatusUdgivet - 2000
Udgivet eksterntJa

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